ワ-ルデンブルグ症候群の聾犬における耳の観察

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The authors used a deaf dog of Waardenburg's syndrome for the study of the ear.Under general anesthesia, part of the head was perfused in vivo with 1% paraformaldehyde and 3% glutaraldehyde. After the removed temporal bones (inner ears) were again perfused with 2% osmic acid from the round window to the oval window, specimens of the membranous labyrinths were prepared by means of the surface preparation technique, to be observed under phase-contrast-and scanning electon-microscopes.The results were, regardless of the existence of heterochromia iridum, as follows: the hair cells of the organ of Corti had all disappeared; that is, total degeneration of the organ of Corti and remnants of supporting cells, especially pillar cells, were observed on the basilar membrane. The cochlear duct had collapsed and Reissner's membrane was adherent to the upper surface of the tectorial membrane. The stria vascularis was found to have atrophied.There was also a noteworthy finding of conspicuous calcification on the upper surface of the tectorial membrane.It appears that this hereditary deaf syndrome is characterized by degeneration of the membranous labyrinths with remarkable calcification of the tectorial membrane. The authors considered its pathological mechanism to be related to hereditary mucopolysaccharidosis.
耳鼻咽喉科臨床学会の論文